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Abstract Background Respiratory involvement defines the clinical outcome of neuromuscular diseases (NMD). The lung clearance index (LCI) is a marker of lung ventilation inhomogeneity and indicates small airway disease. It is determined by mulitple breath washout lung function (MBW). The merit of LCI is undisputed for primary lung diseases like cystic fibrosis, but its role in NMD is unclear. Methods We investigated the role of MBW in patients with NMD and the effect of two different tracer gases and cough assist devices on the LCI. Patients and controls performed MBW with nitrogen (N2) and sulfur hexafluoride (SF6), whereas the latter analysis was repeated after the use of a cough assist device in the NMD group. LCI was compared to forced vital capacity (FVC) and peak cough flow (PCF). Results 24 NMD patients (12 Duchenne Muscular Dystrophy, 8 Spinal Muscular Atrophy, 4 other NMDs) and 15 healthy controls were enrolled. In the NMD group, overall LCI N2 was higher than LCI SF6 (9.67 ± 1.56 vs. 8.71 ± 1.47; mean ± SD; p

Background: Non-invasive ventilation (NIV) has been shown to be the most appropriate therapy for COPD patients with chronic respiratory failure. While physiological parameters and long-term outcome frequently serve as primary outcomes, very few studies have primarily addressed the impact of NIV initiation on sleep quality in COPD. Methods: This single-center prospective cohort study comprised NIV-naïve patients with COPD. All patients underwent polysomnographic evaluation both at baseline and at 3 months follow-up, accompanied by the assessment of health-related quality of life (HRQL) using the Severe Respiratory Insufficiency Questionnaire (SRI) and the Epworth Sleepiness Scale (ESS). A subgroup evaluation was performed to address the impact of comorbid obstructive sleep apnea syndrome (OSAS). Results: Forty-six patients were enrolled and twenty-five patients completed the follow-up period (66.7 ± 7.4 years). NIV resulted in an increase in slow-wave sleep (+2% (−3.5/7.5), p = 0.465) and rapid eye movement sleep (+2.2% (−1.0/5.4), p = 0.174), although no statistical significance could be detected. ESS (−1.7(−3.6/0.1), p = 0.066) also showed a positive trend. Significant improvements in the Respiratory Disturbance Index (RDI) (−12.6(−23.7/−1.5), p = 0.027), lung function parameters, transcutaneous PCO2 and the SRI summary scale (4.5(0.9/8), p = 0.016) were observed. Conclusion: NIV therapy does not decrease sleep quality and is even capable of improving HRQL, transcutaneous PaCO2, daytime sleepiness and RDI, and the latter especially holds true for patients with comorbid OSAS. [ABSTRACT FROM AUTHOR]

Background: The Severe Respiratory Insufficiency Questionnaire (SRI) is a multidimensional instrument for health-related quality of life (HRQL) assessment in patients with chronic respiratory failure (CRF). The SRI has originally been developed in German in 2003, but 15 translated versions have been created during the last 18 years with the exclusion of the Italian translation. Aims of the Study: The present project was aimed at creating an Italian version of the SRI. Methods: Professional forward-translation and back-translation procedures have been provided based on the original German version by independent translators, and this was followed by final reconciliation. Results: The Italian SRI contains 49 items covering 7 different subscales which can summarize to one Summary Score. Conclusions: The Italian SRI is a multidimensional instrument, which can be used for HRQL assessment in Italian-speaking patients with CRF. Validation of the Italian version of the questionnaire is formally required in the future. [ABSTRACT FROM AUTHOR]

Background: Respiratory involvement defines the clinical outcome of neuromuscular diseases (NMD). The lung clearance index (LCI) is a marker of lung ventilation inhomogeneity and indicates small airway disease. It is determined by mulitple breath washout lung function (MBW). The merit of LCI is undisputed for primary lung diseases like cystic fibrosis, but its role in NMD is unclear.Methods: We investigated the role of MBW in patients with NMD and the effect of two different tracer gases and cough assist devices on the LCI. Patients and controls performed MBW with nitrogen (N2) and sulfur hexafluoride (SF6), whereas the latter analysis was repeated after the use of a cough assist device in the NMD group. LCI was compared to forced vital capacity (FVC) and peak cough flow (PCF).Results: 24 NMD patients (12 Duchenne Muscular Dystrophy, 8 Spinal Muscular Atrophy, 4 other NMDs) and 15 healthy controls were enrolled. In the NMD group, overall LCI N2 was higher than LCI SF6 (9.67 ± 1.56 vs. 8.71 ± 1.47; mean ± SD; p < 0.033). In controls, LCI N2 did not differ significantly from LCI SF6 (7.03 ± 0.37 vs. 7.05 ± 0.67; p = 0.882). Both LCI N2 and LCI SF6 were significantly higher in NMD patients as in controls (9.67 ± 1.56 vs. 7.03 ± 0.37, p < 0.001, and 8.71 ± 1.478.65 vs. 7.05 ± 0.67, p < 0.001). In the NMD group, both LCI N2 and LCI SF6 showed a negative correlation to FVC (r = - 0.525; p = 0.008 and r = - 0.526; p = 0.008, respectively) and PCF (r = - 0.590; p = 0.002 and r = - 0.641; p = 0.001, respectively). LCI N2 and LCI SF6 correlated well in the NMD group. LCI SF6 did not change significantly after the use of the cough assist in NMD patients (n = 22; 8.65 ± 1.52 pre vs. 8.79 ± 2.03 post, p = 0.667).Conclusion: Lung involvement of patients with neuromuscular diseases goes beyond weakness of respiratory muscles. MBW with both N2 and SF6 is suitable to detect ventilation inhomogeneity in NMD patients with respiratory impairment. Cough assist devices with low to moderate pressure levels do not immediately improve the LCI. [ABSTRACT FROM AUTHOR]

Background: Non-invasive ventilation (NIV) has been shown to be the most appropriate therapy for COPD patients with chronic respiratory failure. While physiological parameters and long-term outcome frequently serve as primary outcomes, very few studies have primarily addressed the impact of NIV initiation on sleep quality in COPD. Methods: This single-center prospective cohort study comprised NIV-naïve patients with COPD. All patients underwent polysomnographic evaluation both at baseline and at 3 months follow-up, accompanied by the assessment of health-related quality of life (HRQL) using the Severe Respiratory Insufficiency Questionnaire (SRI) and the Epworth Sleepiness Scale (ESS). A subgroup evaluation was performed to address the impact of comorbid obstructive sleep apnea syndrome (OSAS). Results: Forty-six patients were enrolled and twenty-five patients completed the follow-up period (66.7 ± 7.4 years). NIV resulted in an increase in slow-wave sleep (+2% (-3.5/7.5), p = 0.465) and rapid eye movement sleep (+2.2% (-1.0/5.4), p = 0.174), although no statistical significance could be detected. ESS (-1.7(-3.6/0.1), p = 0.066) also showed a positive trend. Significant improvements in the Respiratory Disturbance Index (RDI) (-12.6(-23.7/-1.5), p = 0.027), lung function parameters, transcutaneous PCO2 and the SRI summary scale (4.5(0.9/8), p = 0.016) were observed. Conclusions: NIV therapy does not decrease sleep quality and is even capable of improving HRQL, transcutaneous PaCO2, daytime sleepiness and RDI, and the latter especially holds true for patients with comorbid OSAS. [ABSTRACT FROM AUTHOR]

Background: Patients with chronic respiratory insufficiency suffer from many symptoms together with dyspnea. Objective: We evaluated the association of dyspnea on exercise with other symptoms in patients with chronic respiratory insufficiency due to chronic obstructive pulmonary disease or interstitial lung disease. Design: This retrospective study included 101 patients in Tampere University Hospital, Finland. Dyspnea on exercise was assessed with modified Medical Research Council (mMRC) dyspnea questionnaire, and other symptoms were assessed with Edmonton Symptom Assessment System (ESAS) and Depression Scale (DEPS). The study was approved by Regional Ethics Committee of Tampere University Hospital, Finland (approval code R15180/December 1, 2015). Results: Patients with mMRC 4 (most severe dyspnea) compared with those with mMRC 0?3 reported higher symptom scores on ESAS in shortness of breath (median 8.0 [IQR 6.0?9.0] vs. 4.0 [2.0?6.0], p?0.001), dry mouth (7.0 [4.0?8.0] vs. 3.0 [1.0?6.0], p?

Objectives: Patients with chronic respiratory insufficiency suffer from advanced disease, but their overall symptom burden is poorly described. We evaluated the symptoms and screening of depression in subjects with chronic respiratory insufficiency by using the Edmonton symptom assessment system (ESAS). Methods: In this retrospective study, 226 subjects with chronic respiratory insufficiency answered the ESAS questionnaire measuring symptoms on a scale from 0 (no symptoms) to 10 (worst possible symptom), and the depression scale (DEPS) questionnaire, in which the cut-off point for depressive symptoms is 9. Results: The most severe symptoms measured with ESAS (median [interquartile range]) were shortness of breath 4.0 (1.0-7.0), dry mouth 3.0 (1.0-7.0), tiredness 3.0 (1.0-6.0), and pain on movement 3.0 (0.0-6.0). Subjects with a chronic obstructive pulmonary disease as a cause for chronic respiratory insufficiency had significantly higher scores for shortness of breath, dry mouth, and loss of appetite compared to others. Subjects with DEPS ≥9 reported significantly higher symptom scores in all ESAS categories than subjects with DEPS <9. The area under the receiver operating characteristic curve for ESAS depression score predicting DEPS ≥9 was 0.840 (P <.001). If the ESAS depression score was 0, there was an 89% probability of the DEPS being <9, and if the ESAS depression score was ≥4, there was an 89% probability of the DEPS being ≥9. The relation between ESAS depression score and DEPS was independent of subjects' characteristics and other ESAS items. Conclusions: Subjects with chronic respiratory insufficiency suffer from a high symptom burden due to their advanced disease. The severity of symptoms increases with depression and 4 or more points in the depression question of ESAS should lead to a closer diagnostic evaluation of depression. Symptom-centered palliative care including psychosocial aspects should be early integrated into the treatment of respiratory insufficiency. [ABSTRACT FROM AUTHOR]

Ribeiro Baptista, Bruno, Morgane Faure, Gimbada Benny Mwenge, Capucine Morelot-Panzini, Christian Straus, Thomas Similowski, and Jésus Gonzalez-Bermejo. Feasibility of a hypoxic challenge test under noninvasive ventilation versus oxygen in neuromuscular patients with chronic respiratory insufficiency. High Alt Med Biol. 22:346–350, 2021. Background: The British Thoracic Society recommendations suggest that all patients with an oxygen saturation (SpO2) <85% during a hypoxic challenge test (HCT) should receive supplemental oxygen during air travel. However, neuromuscular patients already using ventilatory support are a specific population and noninvasive ventilation (NIV) during a flight could be an alternative to oxygen for hypoxemia correction, through the augmentation of ventilation. Methods: We conducted a comparative, observational study of neuromuscular patients with chronic respiratory failure, requiring nocturnal mechanical ventilation, who were planning to take a flight. HCT was performed with a ventilated canopy placed over the patient's head or the patient's home ventilator. The positive threshold value chosen for the HCT was <90% SpO2. Results: HCTs were performed on 13 adults with neuromuscular diseases using their home ventilator. Among them, 11 had a positive HCT. For all patients with a positive test, hypoxemia was corrected (SpO2 to >90%) by oxygen therapy (+9 [6–12]%, p = 0.0029). Patient's home ventilator also significantly increased the SpO2 by 8 [7–12]% (p = 0.016). Correction of SpO2 during the HCT was not different between oxygen and NIV. NIV was associated with a significant decrease in pressure, end tidal, carbon dioxide (PetCO2) (−10 [−16 to −7.5] mmHg, p = 0.04). Conclusions: The performance of an adapted HCT in home-ventilated patients with a neuromuscular pathology may be useful in a personalized treatment plan for air travel. NIV can be a new alternative to oxygen therapy for neuromuscular patients planning to take a flight. [ABSTRACT FROM AUTHOR]

Copyright of Studia Pneumologica et Phthiseologica is the property of TRIOS, spol. sr.o. and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

BACKGROUND: Home noninvasive ventilation (NIV) improves disease courses of patients with respiratory insufficiency due to neuromuscular diseases. Data about appropriate ventilator settings for pediatric patients are missing. METHODS: In this retrospective study, ventilator settings of 128 subjects with neuromuscular disease aged 0-17 y with NIV were compared between 4 age groups (< 1 y, 0-5 y, 6-11 y, and 12-17 y). Additionally, correlations of ventilator settings with age and vital capacity were investigated in an ungrouped approach. RESULTS: Ventilator backup rate decreased significantly with age, leading to significant backup rate differences between all groups except the oldest two. Median (interquartile range) backup rates were 36 (11.5), 24 (4), 20 (4), and 20 (3) breaths/min in groups 1-4, respectively. Median [IQR] expiratory positive airway pressures (4 [0.5], 4 [0], 4 [0], 4 [1] cm H2O, respectively) and median [IQR] inspiratory positive airway pressures (12 [1.5], 12 [5], 12 [2.3], and 14 [4] cm H2O, respectively) showed no significant differences. However, correlation analyses indicated an increase of inspiratory positive airway pressure with age and decreasing FVC, as well as an increase of backup rates with decreasing FVC. CONCLUSIONS: Similar NIV settings fit all age groups of pediatric subjects with neuromuscular disease. Thus, we propose an expiratory positive airway pressure of 4-5 cm H2O, an inspiratory pressure delta of 8- 10 cm H2O, and an age-oriented backup rate as a starting point for NIV titration. Patients with advanced disease stages might require slightly higher inspiratory positive airway pressures and backup rates. [ABSTRACT FROM AUTHOR]

Background The need for noninvasive ventilation (NIV) is commonly considered a predictor of poor survival, but life expectancy may vary depending on the underlying disease. We studied the factors associated with decreased survival and end-of-life characteristics in an unselected population of subjects starting NIV. Methods We conducted a retrospective study including 205 subjects initiating NIV from 1/1/2012-31/12/2015 who were followed up until 31/12/2017. Results The median survival time was shorter in subjects needing help with activities of daily living than in independent subjects (hazard ratio (HR) for death 1.7, 95% CI 1.2–2.6, P = 0.008) and was also shorter in subjects on long-term oxygen therapy (LTOT) than in those not on LTOT (HR for death 2.8, 95% CI 1.9–4.3, P

• The Respicheck questionnaire looks for symptoms of respiratory involvement in DM1. • The higher the scores on the Respicheck the worst is the respiratory impairment. • A cut-off of 2 discriminates patients with respiratory impairment. • Patients with the higher scores have a higher risk of respiratory involvement. • Patients with scores below 2 have a lower risk of respiratory involvement. Symptoms of respiratory involvement are frequently present but overlooked by patients with Myotonic Dystrophy type 1 (DM1). A respiratory symptom checklist was designed to test whether a DM-specifically designed checklist to detect symptoms of respiratory involvement (The Respicheck Questionnaire) could help patients be more aware of their respiratory problems, if any, and help clinicians in identifying potential candidates for intervention. The Respicheck questionnaire was administered to 58 consecutive adult-onset patients with genetically determined DM1 who did not complain of respiratory involvement per history at enrollment. Based on respiratory function test results patients were divided into 3 groups: A, (n = 17) having no signs of respiratory involvement; B (n = 13), patients having borderline results on respiratory assessments and having no need for respiratory intervention; C, (n = 28) patients having respiratory impairment requiring intervention. Respiratory test results and Respicheck scores were analyzed. Respicheck total score and subscales correlated positively with global respiratory impairment. Respicheck appears to be able to discriminate between patients having a higher level of respiratory dysfunction from those having a lower risk of respiratory involvement. This might allow to better target efforts and resources in respiratory management in DM1. [ABSTRACT FROM AUTHOR]

Diaphragm pacing allows certain quadriplegic patients to be weaned from mechanical ventilation. Pacing failure can result from device dysfunction, neurotransmission failure, or degraded lung mechanics (such as atelectasis). We report two cases where progressive pacing failure was attributed to deteriorated chest wall mechanics. The first patient suffered from cervical spinal cord injury at age 45, was implanted with a phrenic stimulator (intrathoracic), successfully weaned from ventilation, and permanently paced for 7 years. Pacing effectiveness then slowly declined, finally attributed to rib cage stiffening due to ankylosing spondylitis. The second patient became quadriplegic after meningitis at age 15, was implanted with a phrenic stimulator (intradiaphragmatic) and weaned. After a year hypoventilation developed without obvious cause. In relationship with complex endocrine disorders, the patient had gained 31 kg. Pacing failure was attributed to excessive mechanical inspiratory load. Rib cage mechanics abnormalities should be listed among causes of diaphragm pacing failure and it should be kept in mind that a “good diaphragm” is not sufficient to produce a “good inspiration”.

Background. Mediastinal fetal teratoma can be detected as a mass in the chest during a routine prenatal ultrasound screening. Because of the pressure on mediastinal structures it can be the cause of non-immune hydrops fetalis and polyhydramnion. The development of hydrops fetalis leads to fetal death or premature delivery in most reported cases. Early surgical removal is important, but, the result of treatment depends on the stage of development of mediastinal organs and complications in the postoperative period.

Introduction Children with chronic respiratory insufficiency and mechanical ventilation often experience acute illnesses requiring unscheduled hospitalizations. Health-related quality of life (HRQL) may predict future health care utilization. Methods Participants were 30 days to 22 years old with chronic respiratory insufficiency ( N = 120). Parent-proxies completed global HRQL and general health measures. Outcomes were total health care (emergency department, outpatient, inpatient) and inpatient days over 6 months. Adjusted negative binomial regression estimated the effects of global HRQL and general health on utilization. Results Three quarters of children had any utilization; 32% had hospitalizations. Children with poor/fair global HRQL had 3.7 times more health care days than those with very good/excellent global HRQL. Children with poor/fair global HRQL had 6.3 times more inpatient days than those with very good/excellent global HRQL. Similar relationships existed between general health and utilization. Discussion HRQL was associated with health care and inpatient days. Clinical teams can use HRQL as a marker for utilization risk, enabling potentially earlier intervention, better outcomes, and lower costs. [ABSTRACT FROM AUTHOR]

Background: Congenital central hypoventilation syndrome (CCHS) is a rare genetic disease due to PHOX2B mutations. CCHS patients suffer from many autonomic disorders, dominated clinically by defective ventilatory automatisms. From birth, the life of CCHS patients depends on ventilatory support during sleep, involving a high burden of care. Whether or not this impairs the quality of life of these patients during adulthood remains unknown. Methods: We applied the medical outcome study short form-36 (SF-36) to 12 CCHS patients aged 15-33 (9 women) at the time of their passage from pediatric to adult care. Scores for the SF-36 dimensions were compared to the age- and gender-matched French reference population after transformation into standardized Z-scores. The SF-36 physical component summary score (PCS) and mental component summary score (MCS) were compared to American reference values. Results: Median Z-scores were significantly different from zerofor PF (physical functioning, p = 0.020) and GH (general health perception, p = 0.0342) and for PCS (p = 0.020). The other physical dimensions (RP, role limitation due to physical function; BP, bodily pain) and the mental dimensions (VT, vitality; SF, social functioning; RE, role limitation due to emotional function; MH, mental health) and MCS were not altered. Conclusions: We conclude that, despite the physical constraints imposed by CCHS and its anxiogenic nature, this disease is associated with an impairment of health-related quality of life in young adults that remains moderate. Whatever the underlying explanations, these results convey hope to parents with a child diagnosed with CCHS and for patients themselves. [ABSTRACT FROM AUTHOR]

Diaphragm pacing allows certain quadriplegic patients to be weaned from mechanical ventilation. Pacing failure can result from device dysfunction, neurotransmission failure, or degraded lung mechanics (such as atelectasis). We report two cases where progressive pacing failure was attributed to deteriorated chest wall mechanics. The first patient suffered from cervical spinal cord injury at age 45, was implanted with a phrenic stimulator (intrathoracic), successfully weaned from ventilation, and permanently paced for 7 years. Pacing effectiveness then slowly declined, finally attributed to rib cage stiffening due to ankylosing spondylitis. The second patient became quadriplegic after meningitis at age 15, was implanted with a phrenic stimulator (intradiaphragmatic) and weaned. After a year hypoventilation developed without obvious cause. In relationship with complex endocrine disorders, the patient had gained 31 kg. Pacing failure was attributed to excessive mechanical inspiratory load. Rib cage mechanics abnormalities should be listed among causes of diaphragm pacing failure and it should be kept in mind that a “good diaphragm” is not sufficient to produce a “good inspiration”. [ABSTRACT FROM AUTHOR]

High-flow nasal therapy (HFNT) is a safe and simple way to deliver humidified air and oxygen for respiratory support in infants and children. HFNT is well established in an inpatient setting, but home HFNT lacks evidence. In the current study, we studied characteristics and outcomes of pediatric patients with home HFNT. In a monocentric retrospective analysis of data for 10 years (April 2010-April 2020), patient characteristics from the time point of the first discharge from hospital with home HFNT-treatment and the subsequent course were analyzed. Patients were divided into three HFNT indication groups: (1) bronchopulmonary dysplasia (BPD), (2) upper airway obstruction (UAO), and (3) other indications. Forty patients received home HFNT in the study period. Seventeen patients were treated for BPD, 15 for UAO, and 8 had other indications. Twenty-two patients (55%) were successfully weaned from HFNT (12 [70.6%] BPD, 7 [46.7%] UAO, 3 [37.5%] other), while seven patients (17.5%) died during follow-up (4 BPD, 2 UAO, 1 other). Twenty-three patients (57.5%) required (intermittent) additional oxygen application (14 [82.4%] BPD, 6 [40%] UAO, 4 [50%] other). Weaning success and need for additional oxygen were significantly more probable in BPD patients compared to the UOA group. In conclusion, HFNT plays an increasing role in home treatment of respiratory insufficiency of various etiologies in childhood. It often represents a temporary intervention, especially for children with BPD but might also serve as long-term treatment for children in whom other forms of ventilatory support are not feasible or desired., (© 2022 The Authors. Pediatric Pulmonology published by Wiley Periodicals LLC.)

BACKGROUND: Long-term noninvasive ventilation (NIV) is an effective treatment for patients with chronic respiratory failure due to chest-wall deformity, but it is unknown if the time required for the patient to adjust to long-term NIV depends on whether the NIV is volume-targeted or pressure-targeted. OBJECTIVE: To determine whether volume controlled or pressure controlled NIV is easier to implement in patients with chronic respiratory failure due to chest-wall deformity. METHODS: We randomized 16 ventilator-naïve patients to receive either volume-targeted or pressure-targeted nocturnal NIV. The primary outcome was the number of days needed to successfully establish NIV, defined as adequate adjustment and effective ventilation, as measured with overnight arterial blood gas measurement. RESULTS: Two patients did not tolerate volume NIV, and switched to pressure NIV. NIV was successfully established in both groups after a median 6.0 days. There were no significant differences between the groups at any time point in PaCO2 or PaO2 improvement, nor in changes over time. CONCLUSIONS: There was no significant difference in days needed to successfully establish volume NIV versus pressure NIV in patients with chest-wall deformity. However, two patients switched successfully from volume NIV to pressure NIV, which suggests that they preferred pressure NIV. [ABSTRACT FROM AUTHOR]

Background: The measurement of CO2 partial pressure (PCO2) is of great importance. Former systems of transcutaneous capnometry combining the measurement of oxygen partial pressure (PO2) and PCO2 had their limitations due to skin irritations caused by the heating-up of the sensor and a short application time of 4 h. Objectives: To evaluate for the first time combined monitoring of transcutaneous PCO2 (tcPCO2) and oxygen saturation applying a lower temperature (sensor temperature 42°C) and a new sensor technology in healthy individuals during sleep. Methods: Twenty-nine healthy individuals [12 males, age 35.2 ± 17.0 years, body height: 170.2 ± 12.0 cm (mean ± SD), weight: 76.3 ± 15.8 kg, body mass index 26.5 ± 5.4] were monitored for more than 6 h at night with the TOSCA 500 instrument (Radiometer, Basel, Switzerland). tcPCO2 was continuously monitored and its correlation with selective measured capillary PCO2 values (PcapCO2) was monitored at 0.00 and 4.00 h. Results: At 0.00 h, PcapCO2 was 37.1 ± 5.1 mm Hg and tcPCO2 was 43.4 ± 6.6 mm Hg (p < 0.001). At 4.00 h, PcapCO2 was 37.0 ± 5.6 mm Hg and tcPCO2 was 43.5 ± 5.4 mm Hg (p < 0.001). PcapCO2 and tcPCO2 were positively and significantly correlated (0.00 h: r = 0.5, p < 0.02 and 4.00 h: r = 0.72 and p < 0.001) at both time points. In the course of the night, there was no significant drift in the tcPCO2 values. Conclusion: The investigated system enables stable measurement of tcPCO2 without relevant drift in healthy individuals and does not require recalibration. tcPCO2 is highly suitable as a measure of PcapCO2 because the two parameters are highly correlated and there is no inconvenience to the patient. Copyright © 2010 S. Karger AG, Basel [ABSTRACT FROM AUTHOR]

Aim: To evaluate the clinical application of long-term non-invasive ventilation (NIV) in infants with life-threatening ventilatory failure with regard to: diagnosis, age at initiation, indication for and duration of treatment, clinical outcome and mortality and adverse effects. Patients and methods: The medical records of 18 infants treated in a home setting during a 7-year period were reviewed. The criteria for ventilatory support were: (a) transcutaneous partial pressures of carbon dioxide (TcPCO2) >6.5 kPa and oxygen (TcPO2) < 8.5 kPa and (b) decreased cough ability and/or recurrent chest infections. Results: The median age at initiation was 4 months (range 1–12). NIV was initiated because of hypoventilation in 12 infants and because of reduced cough ability and/or recurrent infections in six infants. Tracheotomy was eventually needed in two infants. The median duration of treatment was 24 months (range 1–84). NIV produced significant improvements, with median TcPCO2 falling from 9.9 to 6.1 kPa, and median TcPO2 rising from 9.8 to 11.1 kPa. Conclusion: NIV can be successfully and safely used in infants with prolonged life-threatening ventilatory failure, potentially avoiding intubation and tracheotomy. [ABSTRACT FROM AUTHOR]

Abstract: Purpose: To describe the epidemiological, physiopathological, clinical and therapeutic knowledge concerning cystic fibrosis (CF). Important modifications in the health organization of the care concerning this orphan disease have been implemented in France. The life expectancy has dramatically increased, as well as the knowledge concerning the pathological structure and function of the CFTR gene and protein. This will lead to the development of emerging drug treatments for this lethal disease. Current knowledge and key points: The life expectancy is predicted to exceed 40 years for children born in the 2000s. As a result, there has been a tremendous growth of the adult population that reached 40% of the overall approximately 5000 patients included in the CF French registry (Observatoire National de la Mucoviscidose). Lung disease remains the primary cause of morbidity and mortality. The characteristic phenotypic presentation associates bronchial and rhinosinusal symptoms, pancreatic insufficiency and liver disease. Bronchial damage leads to progressive chronic respiratory insufficiency. Diabetes mellitus and osteoporosis frequently appears in adulthood. Neonatal screening has been implemented in France since 2002. It will prevent delayed diagnosis and its deleterious consequences. Some atypical cases of CF presenting only with one or two organ system involvement can be diagnosed in adulthood. Isolated chronic rhinosinusitis, bronchiectasis, congenital bilateral absence of vas deferens, recurrent pancreatitis, allergic bronchopulmonary aspergillosis, and some case of cholangitis may so revealed late form of cystic fibrosis. Future prospects and projects: The health care is organized in cystic fibrosis centres. Despite gene discovery, treatment still remains symptomatic, based on intensive pulmonary and nutritional treatments. Challenges for new treatments are to correct the basic defect, either by gene therapy or by pharmacological modulation of the abnormal physiological processes. [Copyright &y& Elsevier]

Copyright of EMC-Medecine is the property of Elsevier B.V. and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

It was already documented that acute hypoxemia reduces the oxidative stress following static as well as dynamic handgrip bouts in humans. Then, we examined if chronic hypoxemia could produce the same effect in patients suffering from chronic respiratory insufficiency. In rats, we studied the respective consequence of a one-month exposure to normobaric hypoxia on two muscles (soleus, SOL, and extensor digitorum longus, EDL) which have high and low aerobic metabolism, respectively. Compared to healthy humans, the resting level of erythrocyte reduced glutathione (GSH) was significantly lower in chronic hypoxemic patients, and after a handgrip contraction sustained at 50% of maximal until exhaustion the GSH level and plasma thiobarbituric acid reactive substances (TBARS) did not vary. A 20-min period of oxygen supplementation partly restored the post-handgrip oxidative stress. Compared to control rats, SOL muscle of hypoxemic animals had lower intra-muscular resting level of GSH; after a 3-min muscle stimulation (MS) leading to fatigue, TBARS did not vary in SOL and EDL and the GSH decrease was absent in SOL whereas it persisted in EDL. We concluded that chronic hypoxemia depressed the fatigue-induced oxidative stress, the effects prevailing in muscles having a high oxygen demand. [Copyright &y& Elsevier]

Background: Chronic respiratory insufficiency is mainly the result of ventilatory dysfunction due to various causes, which results in decreased respiratory function. The most important cause of chronic respiratory insufficiency is chronic obstructive pulmonary disease, which has a high incidence and places a great burden on families and society. Therefore, it is important to find an effective treatment for this disease., Methods: This study was a retrospective self-controlled study. Thirty-eight patients receiving home oxygen therapy who visited the outpatient oxygen therapy clinic from November 2020 to August 2021 were selected. After a comprehensive and systematic diagnosis and treatment, home oxygen therapy as standardized, and 38 patients were followed up for 6 months. Oxygen therapy compliance, the average daily oxygen therapy duration, arterial blood gas analysis, activities of daily living (ADL), and modified Medical Research Council Dyspnea Scale (mMRC) scores before and 6 months after outpatient oxygen therapy intervention were compared. The number of hospital visits for acute exacerbation of chronic respiratory insufficiency and the medical costs incurred within 6 months before and after the outpatient oxygen therapy intervention were also compared., Results: After 6 months of outpatient oxygen therapy intervention, the compliance with oxygen therapy was significantly improved, the average daily oxygen therapy duration was significantly prolonged, the oxygen saturation (SaO2) and partial pressure of oxygen (PaO2) values were significantly increased, and the mMRC score was significantly decreased (P<0.05), while the differences in the ADL scores were not statistically significant (P>0.05). The number of hospital visits and the medical expenses due to acute exacerbation of chronic respiratory insufficiency within 6 months after the outpatient oxygen therapy intervention were significantly lower than those in the 6 months before the intervention (P<0.05)., Conclusions: As a relatively new clinical service in China, outpatient oxygen therapy can improve the efficacy of home oxygen therapy for patients with chronic respiratory insufficiency through patient assessment, personalized selection of oxygen therapy equipment, and standardization of the implementation of home oxygen therapy. It provides an economically valuable treatment.

The efficacy and safety of almitrine bismesylate, a new respiratory stimulant, in patients with the hypoxaemic form of chronic respiratory insufficiency caused by chronic bronchitis and emphysema has been assessed. The multicentre trial of 12 weeks duration was double-blind and placebo controlled, with individual and group comparisons. Twenty three patients received almitrine 50 mg b.d. p.o. and 17 took placebo. In the almitrine group a significant increase in PO was achieved (control value 54.4 mm Hg, rising to 59.1 mm Hg after 6 weeks, and to 59.4 mm Hg after 12 weeks). There was also a significant decrease in PCO in the almitrine group after 12 weeks. No correlation was found between the plasma almitrine concentration, PO and PCO. Lung function (FVC, FEV, FEV/FVC, Raw, TLC, RV, FRC) did not change in either group, but the degree of dyspnoea and performance in the 6 min walking test were significantly improved in the almitrine group. Adverse reactions appeared in 6 out of 23 patients on almitrine bismesylate (headache, urticaria, breathlessness, diarrhoea, chest pain, nausea and vomiting), causing drop out of 4 patients. Thus, almitrine bismesylate can be considered useful in the treatment of patients with chronic respiratory insufficiency. [ABSTRACT FROM AUTHOR]